HAEM4:Splenic Marginal Zone Lymphoma: Difference between revisions

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<blockquote class='blockedit'>{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].
<blockquote class="blockedit">{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].
}}</blockquote>
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==Primary Author(s)*==
==Primary Author(s)*==
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==Definition / Description of Disease==
==Definition / Description of Disease==


*Marginal zone lymphomas (MZL) make up 7% of non-Hodgkin lymphomas. The three subtypes are: MALT (70%), splenic (20%), and nodal (10%). '''Splenic marginal zone lymphoma (SMZL)''' is an Indolent mature B-cell neoplasm of adults
*Indolent mature B-cell neoplasm of adults involving the spleen, blood, and bone marrow
*Indolent mature B-cell neoplasm of adults involving the spleen, blood, and bone marrow
*Splenic white pulp effacement by small lymphocytes and pale marginal zone involved by larger cells
*Splenic white pulp effacement by small lymphocytes and pale marginal zone involved by larger cells
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*Males ~ Females
*Males ~ Females


==Clinical Features<ref name=":2" /><ref name=":6" />==
==Clinical Features<ref name=":2" /><ref name=":6" /><small><sup>[3]</sup></small>==
'''Signs & Symptoms'''
'''Signs & Symptoms'''


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|7q31-32||Loss (heterozygous)
|7q31-32||Loss (heterozygous)
|Unknown; possible haploinsufficiency of IRF5 tumor suppressor<ref>{{Cite journal|last=Fresquet|first=Vicente|last2=Robles|first2=Eloy F.|last3=Parker|first3=Anton|last4=Martinez-Useros|first4=Javier|last5=Mena|first5=Maria|last6=Malumbres|first6=Raquel|last7=Agirre|first7=Xabier|last8=Catarino|first8=Susana|last9=Arteta|first9=David|date=2012|title=High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09226.x|journal=British Journal of Haematology|language=en|volume=158|issue=6|pages=712–726|doi=10.1111/j.1365-2141.2012.09226.x}}</ref>||26–45%<ref name=":2">{{Cite journal|last=Salido|first=Marta|last2=Baró|first2=Cristina|last3=Oscier|first3=David|last4=Stamatopoulos|first4=Kostas|last5=Dierlamm|first5=Judith|last6=Matutes|first6=Estela|last7=Traverse-Glehen|first7=Alexandra|last8=Berger|first8=Francoise|last9=Felman|first9=Pascale|date=2010|title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group|url=https://ashpublications.org/blood/article/116/9/1479/103841/Cytogenetic-aberrations-and-their-prognostic-value|journal=Blood|language=en|volume=116|issue=9|pages=1479–1488|doi=10.1182/blood-2010-02-267476|issn=0006-4971}}</ref><ref name=":3">{{Cite journal|last=Baró|first=Cristina|last2=Salido|first2=Marta|last3=Espinet|first3=Blanca|last4=Astier|first4=Laura|last5=Domingo|first5=Alicia|last6=Granada|first6=Isabel|last7=Millà|first7=Fuensanta|last8=Carrió|first8=Ana|last9=Costa|first9=Dolors|date=2008|title=New chromosomal alterations in a series of 23 splenic marginal zone lymphoma patients revealed by Spectral Karyotyping (SKY)|url=https://linkinghub.elsevier.com/retrieve/pii/S0145212607003505|journal=Leukemia Research|language=en|volume=32|issue=5|pages=727–736|doi=10.1016/j.leukres.2007.09.012}}</ref><ref name=":4">{{Cite journal|last=Gruszka-Westwood|first=Alicja M.|last2=Hamoudi|first2=Rifat|last3=Osborne|first3=Lucy|last4=Matutes|first4=Estella|last5=Catovsky|first5=Daniel|date=2003|title=Deletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes|url=http://doi.wiley.com/10.1002/gcc.10142|journal=Genes, Chromosomes and Cancer|language=en|volume=36|issue=1|pages=57–69|doi=10.1002/gcc.10142|issn=1045-2257}}</ref>
|Unknown; possible haploinsufficiency of IRF5 tumor suppressor<ref>{{Cite journal|last=Fresquet|first=Vicente|last2=Robles|first2=Eloy F.|last3=Parker|first3=Anton|last4=Martinez-Useros|first4=Javier|last5=Mena|first5=Maria|last6=Malumbres|first6=Raquel|last7=Agirre|first7=Xabier|last8=Catarino|first8=Susana|last9=Arteta|first9=David|date=2012|title=High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09226.x|journal=British Journal of Haematology|language=en|volume=158|issue=6|pages=712–726|doi=10.1111/j.1365-2141.2012.09226.x}}</ref>||26–45%<ref name=":2">{{Cite journal|last=Salido|first=Marta|last2=Baró|first2=Cristina|last3=Oscier|first3=David|last4=Stamatopoulos|first4=Kostas|last5=Dierlamm|first5=Judith|last6=Matutes|first6=Estela|last7=Traverse-Glehen|first7=Alexandra|last8=Berger|first8=Francoise|last9=Felman|first9=Pascale|date=2010|title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group|url=https://ashpublications.org/blood/article/116/9/1479/103841/Cytogenetic-aberrations-and-their-prognostic-value|journal=Blood|language=en|volume=116|issue=9|pages=1479–1488|doi=10.1182/blood-2010-02-267476|issn=0006-4971}}</ref><ref name=":3">{{Cite journal|last=Baró|first=Cristina|last2=Salido|first2=Marta|last3=Espinet|first3=Blanca|last4=Astier|first4=Laura|last5=Domingo|first5=Alicia|last6=Granada|first6=Isabel|last7=Millà|first7=Fuensanta|last8=Carrió|first8=Ana|last9=Costa|first9=Dolors|date=2008|title=New chromosomal alterations in a series of 23 splenic marginal zone lymphoma patients revealed by Spectral Karyotyping (SKY)|url=https://linkinghub.elsevier.com/retrieve/pii/S0145212607003505|journal=Leukemia Research|language=en|volume=32|issue=5|pages=727–736|doi=10.1016/j.leukres.2007.09.012}}</ref><ref name=":4">{{Cite journal|last=Gruszka-Westwood|first=Alicja M.|last2=Hamoudi|first2=Rifat|last3=Osborne|first3=Lucy|last4=Matutes|first4=Estella|last5=Catovsky|first5=Daniel|date=2003|title=Deletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes|url=http://doi.wiley.com/10.1002/gcc.10142|journal=Genes, Chromosomes and Cancer|language=en|volume=36|issue=1|pages=57–69|doi=10.1002/gcc.10142|issn=1045-2257}}</ref>
|-
|17p13 (TP53)
|Loss
|
|3%–17%
|-
|-
|3/3q||Gain (trisomy)
|3/3q||Gain (trisomy)
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==Notes==
==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.
[[Category:HAEM4]] [[Category:DISEASE]]
[[Category:HAEM4]]  
[[Category:DISEASE]]